Extra-adrenal Pheochromocytoma: Experience in Mulago Hospital

Exta-adrenal pheochromocytomas are rare tumors that arise from extra-adrenal chromaffin cells of the sympathetic ganglia. Experience with two cases is reported here and a review of literature was conducted. Like pheochromocytomas, extra-adrenal pheochromocytomas present with episodic hypertension, tachycardia, headache, and diaphoresis, and can be either benign or malignant. Diagnosis is made by serum and urine analysis for catecholamines and metanephrines, and confirmed with imaging studies including computed tomography scanning, magnetic resonance imaging, or 123-I metaiodobenzylguanidine imaging. Ultrasound scanning in the developing world is beneficial. Genetic testing should be offered were available, particularly patients who are young, have multiple tumors, or have a family history of malignancy. Management of extra-adrenal pheochromocytoma is enblock en-mass surgical resection. Chemotherapy, and radiation therapy may be necessary in malignant disease. Long-term follow-up is essential, as extra-adrenal pheochromocytomas can recur many years after initial diagnosis.